Histopathologic and immunohistochemical analysis supported the diagnosis of poorly differentiated synovial sarcoma, which was further. The poorly differentiated variant, in particular, may be histologically indistinguishable from other small round cell tumors. Immunohistochemistry ihc was positive for vimentin, cd 99, bcl2, mic2 and focally for ema and negative for cd 34, ck, desmin, synaptophysin, and wt1. Synovial sarcoma can be morphologically classified into three main categories.
Synovial sarcoma has fusion of ss18 with ssx1, ssx2 or ssx4. Pathology clinic synovial sarcoma lester dr thompson, md. Dumbbell shaped poorly differentiated pelvic synovial. Diagnosis of synovial sarcoma of the pleura and differentiation. Poorly differentiated synovial sarcoma in the wrist case. This is the first extensive description of the cytologic features of this neoplasm. Mar 10, 2006 cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and ewing sarcoma. Six tumors were classified histologically as poorly differentiated, while 39 were described as monophasic and 10 as biphasic. Poorly differentiated areas in synovial sarcomas ss are known. Synovial sarcoma, monophasic fibrous and poorly differentiated. Intrathoracic synovial sarcomas show a high proportion that are poorly differentiated. Sep 10, 2015 the exact underlying cause of synovial sarcoma is poorly understood.
Primary pulmonary and mediastinal synovial sarcoma. We report here a mouse model of synovial sarcoma based on conditional expression of the human sytssx2. In contrast to synovial sarcoma, the sarcomatoid components of both malignant mesothelioma and sarcomatoid carcinoma usually show considerable nuclear atypia. Sarcomatoid carcinoma and biphasic malignant mesothelioma should be differentiated from each other, as well as from biphasic synovial sarcoma.
We studied a case arising in the pleural cavity of a young subject, characterised by the presence of spindle cell, small cell. According to the guidelines of the adasp, synovial sarcoma is not graded although it often metastasizes. Poorly differentiated synovial sarcoma is associated with. The immunohistochemical findings were consistent with the diagnosis of a poorly differentiated synovial sarcoma, arising from the vaginal wall. Synovial sarcoma ss is a clinically and histologically welldefined entity. Pdf poorly differentiated primary synovial sarcoma of.
However, our patient had poorly differentiated synovial sarcoma. Histopathological and immunohistochemical findings. Poorly differentiated monophasic synovial sarcoma of the. Monomorphic spindled variants, as well as the less common poorly differentiated variants, may be confused with other softtissue sarcomas. Sep 01, 2003 read identification of poorly differentiated synovial sarcoma. A child recently presenting with synovial sarcoma of the postauricular. In a few tumors, calcification is extensive, and the improved prognosis in such cases 406 cyril fisher mineralization or osteochondroid metaplasia required to define either calcifying or ossifying ss remains unde termined. Read utility of cytokeratin subsets for distinguishing poorly differentiated synovial sarcoma from peripheral primitive neuroectodermal tumour, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips.
Mitoses are uncommon in synovial sarcoma except for poorly differentiated variants. Rare tumor in lung associated with chest pain, hemoptysis, dyspnea, cough, fever rarely is cystic am j surg pathol 2010. The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of tx. This patient presented 11 months later with ipsilateral axillary lymph node metastasis, which emphasizes the unfavorable prognosis of this synovial sarcoma variant. It typically occurs in younger patients, with the peak incidence in the third fourth decade and most cases show at least focal cytokeratin expression. Patients with synovial cell sarcoma are often between the ages of 15 and 35 years old. These may include muscle, fat, blood or lymph vessels. Synovial sarcoma ss is a rare malignant neoplasm that arises most commonly in joint capsules and articular tendons, but its relationship to the synovium is not always obvious. Poorly differentiated monophasic synovial sarcoma of the mediastinum. Microscopic examination of this case revealed a highgrade, biphasic, poorly differentiated synovial sarcoma with abundant necrosis and mitoses more than 1010 highpower fields.
Poorly differentiated synovial sarcoma of the vagina. Focused synovial sarcoma with stained slides of pathology. Synovial cell sarcoma is a rare and poorly understood canine tumor entity. Synovial sarcoma ss is a neoplasm that poses diagnostic problems, due to its histologic heterogeneity.
Synovial sarcomapowerpoint presentation new authorstream. We report a case of a 40 year old male patient presented by a mass in the left lung which proved to be a poorly differentiated primary synovial sarcoma. They are termed ss because of their histologic resemblance to the synovium, but they rarely involve a synovial. Definitiondiagnostic criteria ss is a soft tissue sarcoma usually divided into three subtypes. Sarcoma histopathology classification has itself proven to be difficult, since in addition to the great histological heterogeneity, the problem in studying sarcomas is further compounded by the fact that numerous histological subtypes exist. Poorly differentiated synovial sarcoma is a diagnostically challenging neoplasm. Synovial sarcoma develops in cells around joints and tendons. Synovial sarcoma, a primary liver tumor a case report. Synovial sarcoma derives its name from the fact that microscopically it resembles normal. Synovial sarcoma, abbreviated ss, is an uncommon malignant soft tissue tumour, typically seen in young adults. The presentation is most often with a slowlyenlarging soft tissue mass which may have been noted for some years and gives a false impression of a benign process 4. Poorly differentiated tumors are characterized by necrosis and increased mitotic figures. Synovial sarcoma, nos 90403 synovial sarcoma, nos 904 synovial sarcoma, spindle cell. Synovial sarcoma is a soft tissue tumor occurring in the biphasic or monophasic type and consisting of epithelial andor spindle cell components.
Jul 29, 20 histopathology and immunohistochemistry confirmed synovial sarcomas with poorly differentiated cells. Introduction pulmonary synovial sarcomas represent 0. Poorly differentiated variant of synovial sarcoma is a round cell sarcoma which may be histologically indistinguishable from ewings sarcoma pnet the diagnosis of synovial sarcoma was established by. Synovial sarcoma high quality pathology images of soft tissue. Low and high power images of poorly differentiated variant of synovial sarcoma. Synovial sarcoma international journal of contemporary medical. Grading staging report increasing amount of poorly differentiated area indicates a worse prognosis. Ss is a soft tissue sarcoma usually divided into three subtypes. Synovial sarcoma appears to arise from still poorly characterized immature mesenchymal progenitor cells through the action of its primary oncogenic driver, the ss18ssx fusion gene, which encodes. One case report of fibrosarcoma with translocation tx. Histology description histology behavior histology behavior description c000c006,c008c009 lip 800 neoplasm.
However, the distinction between poorly differentiated synovial sarcoma and fibrosarcoma may be difficult and could represent a histological variant of synovial sarcoma. A child recently presenting with synovial sarcoma of the postauricular region and the limited. Adverse outcome may also be related to the high rate of poorly differentiated morphology no known differences are noted in the clinical behavior of monophasic and biphasic synovial sarcoma. Synovial sarcomas occur most commonly in young patients, representing about 10% of soft tissue sarcomas in all age groups and about 1520% in adolescents, with more. Synovial sarcoma with relevant immunocytochemistry and. The tumor, which was located in the right upper lobe, was lobulated, relatively wellcircumscribed, and whitish to yellowish in color. Pdf poorly differentiated synovial sarcoma in the wrist. It affects soft tissues, which connect, support, and surround bones and organs in your body. Despite differing opinions regarding classification of synovial sarcoma, the histopathology is the same for those arisingin the headand neck and those arisingin the extremities. Many times the histology of these tumors looks like metastatic carcinoma, malignant melanoma, epithelioid sarcoma and malignant schwannoma raising the need of immunohistochemistry for differential diagnosis.
Synovial sarcoma, an aggressive soft tissue tumor with high rate of local recurrence and distant metastasis, is currently thought to originate from mesenchymal stem cells. Synovial sarcoma surgical pathology criteria stanford. Utility of cytokeratin subsets for distinguishing poorly. He stain has an epithelioid growth pattern and relatively uniform round cells, a characteristic that makes differentiation from other small round blue cell tumors eg, ewing sarcoma difficult without immunohistochemical staining.
We studied a case arising in the pleural cavity of a young subject, characterised by the presence of spindle cell, small cell, and large epithelioid cell areas. Primary poorly differentiated monophasic synovial sarcoma of. Synovial sarcoma, histologically mimicking primitive. Present study does not show any evidence of necrosis. Synovial sarcomas are usually found in the arms or legs around a joint capsule. Necrosis is uncommon in synovial sarcoma but encountered in recurrent or metastatic diseases or cases with therapeutic irradiation.
Poorly differentiated synovial sarcoma in the wrist case report. Imaging of synovial sarcoma with radiologicpathologic. Primary intraprostatic synovial sarcoma archives of. Synovial sarcoma symptoms, causes, diagnosis, treatment. We present a case of a 16yearold female with a poorly differentiated synovial sarcoma, where the diagnosis was established by molecular diagnostic techniques, including reverse transcriptase polymerase chain reaction rtpcr for detecting the ss18ssx2 fusion transcript, and fluorescence in situ hybridization fish for demonstrating the. Sarcomatoid carcinoma an overview sciencedirect topics. Histopathology was suggestive of poorly differentiated synovial sarcoma.
Department of pathology, the first affiliated hospital of sun yatsen university. Poorly differentiated synovial sarcomas are high cellular. To our knowledge, this is the first report of a poorly differentiated variant of synovial sarcoma occurring in dura mater and confirmed by cytogenetic analysis. Poorly differentiated synovial sarcoma is a rare soft tissue tumor. Two cell types can be seen microscopically in synovial sarcoma. A comparison of clinicopathological and cytogenetic features with those of typical synovial sarcoma article in histopathology 433. Tissue microarray validation of epidermal growth factor. Also, distinction from malignant peripheral nerve sheath tumors may be difficult, as both may. However, studies show that a certain genetic change is identified in more than 90% of cases. Poorly differentiated variant of synovial sarcoma is a round cell sarcoma which may be histologically indistinguishable from ewings sarcoma pnet. Three main types of poorly differentiated synovial sarcoma were observed and included tu mors with. The initial histology showed spindle cell hypercellular proliferation in fascicular pattern, in which the spindled tumour cells have ample eosinophilic cytoplasm that resembled smooth.
Apr 10, 2007 synovial sarcoma is an aggressive softtissue malignancy marked by a unique tx. Begueret et al 4 reported that almost 40% of primary pulmonary and mediastinal synovial sarcomas were poorly differentiated. Because of this new definitional criterion, the histologic features described may in the future be expanded see poorly differentiated pattern description. General information synovial sarcoma is a common soft tissue malignancy accounting for 5 10 % of soft tissue sarcomas. Synovial sarcoma, for example, was initially recognized as a biphasic tumor with epithelial and uniform spindlecell components, but now encompasses a wider morphological spectrum of tumors, including monophasic spindle cell and poorly differentiated subtypes, 1,2 such that classic biphasic tumors now account for a minority of cases of synovial.
We describe a case of a poorly differentiated monophasic synovial sarcoma arising in the lung of a 50yearold man. Cluster analysis of immunohistochemical profiles in synovial. The clinical characteristics of our 55 synovial sarcoma cases and the results of immunostaining are summarized in additional file1. This tumor type accounts for about 10% of all soft tissue sarcomas and is subclassified into three subtypes. The biphasic type contains epithelial and spindle cell elements, which occur in varying proportions. The diagnosis of synovial sarcoma was established by. The poorly differentiated subtype accounts for about 20% of sss and shows a more aggressive clinical behavior than the other subtypes. Synovial sarcoma genetic and rare diseases information. Using this model, we have identified myoblasts as a potential source of synovial sarcoma. Identification of poorly differentiated synovial sarcoma. Histopathology and immunohistochemistry confirmed synovial sarcomas with poorly differentiated cells. Primary poorly differentiated monophasic synovial sarcoma of ileum.
All soft tissue tumors with this translocation are considered synovial sarcomas. Jul 31, 20 dermatopathology reference describes poorly differentiated synovial sarcoma histopathology including histologic features and provides links to additional medical references. Synovial sarcomas are relatively rare soft tissue tumors usually involving the extremities of young. More specifically, a translocation between chromosome x and chromosome 18 appears to play a role in the development of synovial.
We performed stains for mucosubstances and analysed the expression of cytokeratins 56, 7, 8, 18, 19, cea, cd34, berep4 and calretinin to characterize the phenotype. One fibrous type, known as a spindle or sarcomatous cell, is relatively small and uniform, and found in sheets. We present a case with primary superficial cutaneous synovial sarcoma without. Synovial sarcoma is a relatively common sarcoma in adults, which in its classic bimorphic form infrequently poses a diagnostic problem.
Synovial sarcomas are relatively common intermediatetohigh grade malignant soft tissue tumours, often with an initial indolent course, affecting young patients, and most commonly involving the soft tissue surrounding the knees. Due to equivocal ihc fi ndings molecular analysis was done which confi rmed the diagnosis as synovial sarcoma. University department of pathology, western infirmary, glasgow g11. Synovial sarcoma powerpoint presentation new authorstream presentation. It is not associated with pax3 rearrangements, a typical finding in alveolar rhabdomyosarcoma and biphenotypic sinonasal sarcoma. The indolent growth pattern of this sarcoma justifies the well circumscribed. A poorly differentiated synovial sarcoma sytssx1 expresse. Percent poorly differentiated area should be reported. Printable synovial sarcoma surgical pathology criteria. Synovial sarcoma is a welldefined entity that constitutes approximately 10% of all soft tissue sarcomas. Synovial sarcoma is a malignant soft tissue tumor representing 5.
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